MS is an immune-mediated, progressive demyelinating disease of the CNS. Demyelination is the destruction or loss of the myelin, a fatty and protein material that surrounds certain nerve fibers in the brain and spinal cord, resulting in impaired nerve impulse transmission. Its peak of onset is from 20 to 50 years of age, but can occur at any age. It is found in women three times more often than in men. Its cause is unknown. There are several acute and subacute forms of MS. Less severe forms include RIS and CIS. The four main clinical forms are Remitting-Relapsing MS (RRMS), Secondary Progressive MS, Primary Progressive MS (PPMS), and Progressive-Relapsing MS.
- Radiologically Isolated Syndrome (RIS): MRI imaging finds lesions due to MS, but the patient presents no symptoms. Often, MS is diagnosed within five years after initial findings of lesions.
- Clinically Isolated Syndrome (CIS): presence of acute or subacute findings for at least 24 hours.
Pathophysiology
- Sensitized T and B lymphocytes cross the blood-brain barrier to check the CNS for antigens and leave.
- In MS, Sensitized T cells remain in the CNS and promote the infiltration of other agents that damage the immune system.
- Damage results in inflammation, destroying mostly white matter in the CNS myelin and oligodendroglial cells that produce myelin in the CNS.
- Demyelination interrupts the flow of nerve impulses.
- Plaques appear on demyelinated axons, further interrupting nerve transmission. The most frequently affected areas are the optic nerves, chiasm, and tracts; the cerebrum; the brainstem and cerebellum; and the spinal cord.
- The axons begin to degenerate, resulting in permanent and irreversible damage.
Clinical Manifestations
The course of MS ranges from being asymptomatic or having benign, mild presentation:
- Radiologically Isolated Syndrome: no symptoms
- Clinically Isolated Syndrome: unilateral optic neuritis, focal symptoms, or partial myelopathy
- Remitting-Relapsing MS: consists of 85% of patients. Every relapse features complete recovery but produces residual deficits. These deficits accumulate overtime between relapses, contributing the functional decline. This may progress to secondary progressive form, where progression of disease occurs with or without relapses.
- Primary Progressive MS: consists of 15% of patients. Disabling symptoms steadily increase, with rare plateaus and temporary minor improvement. This may result in quadriparesis, cognitive dysfunction, visual loss, and brainstem syndromes.
- Progressive-Relapsing MS is the rarest presentation, found in 5% of cases. Relapses occur similar to RRMS, but disabling progression continues between relapses.
Signs and symptoms vary, reflecting the location of the plaques or lesions in the brain. Physical, emotional, and cognitive symptoms can impact the quality of life.
- Fatigue, depression, weakness, numbness, dystaxia, loss of balance, spasticity, and pain are all common.
- Visual disturbances from lesions in the optic nerves or their connections: blurring of vision, diplopia, scotoma (patchy blindness), and total blindness.
- Fatigue affects the most people with MS, and is often the most disabling symptom. Heat, depression, anemia, deconditioning, and medication may contribute to fatigue. Resolution of these contributing factors can help with fatigue.
- Pain is another common symptom. Lesions on the sensory pathways cause pain, along with other sensory manifestations including paresthesias, dysesthesias, and proprioception loss. Daily analgesic medication (opioids, anticonvulsants, antidepressants) may be required. In severe cases, pain pathways can be surgically interrupted.
- In perimenopausal women, pain may be related to osteoporosis resulting from estrogen loss, immobility, and corticosteroid therapy. Bone Mineral Density Testing is recommended.
- Spasticity is found in 90% of MS cases and is characterized by muscle hypertonicity with increased resistance to stretch associated with weakness, increased DTRs, and decreased superficial reflexes. These result from involvement of the pyramidal tracts, the main motor pathways of the spinal cord.
- Cognitive and psychosocial problems may reflect involvement of the frontal or parietal lobes. Some degree of cognitive change (memory loss, impaired concentration) is found in half of patients.
- Ataxia and Tremors may result from involvement of the cerebellum or basal ganglia.
- Emotional lability and euphoria may result from the loss of control connections between the cortex and the basal ganglia.
- Bladder, bowel, and sexual dysfunctions are common. UTIs, constipation, pressure injuries, contracture deformities, dependent pedal edema, pneumonia, osteoporosis, and emotional, social, marital, economic, and vocational problems may also occur.
Diagnostic Examination
Diagnosis is based on clinical, imaging, and laboratory findings. The presence of plaques in the CNS disseminated in space and over time observed on MRI scans with no better explanation for the clinical presentation.
- Electrophoresis of CSF is done to determining immune system abnormality (multiple bands of IgG bonded together).
- Evoked Potential Studies can help define the extent of the disease process and monitor changes.
- Urodynamic Studies determine any underlying bladder dysfunctions.
- Neuropsychological Testing may be used to assess cognitive impairment.
- Sexual history defines changes in sexual function.
Management
- Medical: there is no cure for MS. An individual treatment program provides symptomatic relief and support, especially for patients with cognitive impairment. Goals are to (a) delay the progression of the disease, (b) manage chronic symptoms, and (c) treat acute exacerbations. Symptoms requiring intervention include ataxia, bladder dysfunction, depression, fatigue, and spasticity.
- Pharmacologic:
- Disease-Modifying therapies reduce the frequency or duration of relapse, and the number and size of plaques observed on MRI in RRMS. The same therapies are not effective in PPMS.
- Interferon beta-1a and Interferon beta-1b SQ q2d, another preparation of interferon beta-1a IM q1w, and pegylated interferon beta-1a SQ q2w.
- Side effects of all interferon medication includes flulike symptoms, increased liver function tests, leukopenia, headache, depression, and skin necrosis.
- Glatiramer acetate reduces the frequency of relapse in RRMS, administered SQ od. Adverse effects include injection-site reactions and flushing, but are often self-limiting. These have no monitoring parameters.
- Teriflunomide, fingolimod, and dimethyl fumarate are oral disease-modifying therapies that may be better tolerated by patients who have difficulty with injection reactions.
- Ocrelizumad reduce annual relapses by 6% in patients with PPMS.
- IV methylprednisolone is used to treat acute exacerbations, shortening the duration of relapse, but have no long term benefit. Anti-inflammatory effects act on T cells and cytokines. It is given 1g IV od for 3 to 5 days, followed by an oral taper of prednisone. Side effects include mood swings, weight gain, and electrolyte imbalances.
- IV Mitoxantrone q3mo can reduce clinical relapse frequency in patients with worsening RRMS (secondary progressive form). It has an adverse side effect of cardiac toxicity, and has a set lifetime maximum dose.
- Interferon beta-1a and Interferon beta-1b SQ q2d, another preparation of interferon beta-1a IM q1w, and pegylated interferon beta-1a SQ q2w.
- Symptom Managing therapies
- Disease-Modifying therapies reduce the frequency or duration of relapse, and the number and size of plaques observed on MRI in RRMS. The same therapies are not effective in PPMS.
| Symptoms | Medication |
|---|---|
| Spasticity | Baclofen oral or intrathecal (for severe spasticity), a gamma-aminobutyric acid (GABA) agonist, treats spasticity. |
| Spasticity, Debilitated Motor Function | Benzodiazepines (Diazepam), Tizanidine, and Dantrolene may also be used to treat spasticity and improve motor function. |
| Fatigue | Amantadine, Pemoline, Dalfamprindine can help with debilitating fatigue. |
| Ataxia | Beta-adrenergic Blockers (Propanolol), Gabapentin (an anticonvulsant), and Benzodiazepines (Clonazepam) is used to treat ataxia. |
| Bladder and Bowel Problems | Anticholinergic agents, alpha-adrenergic blockers, antispasmodic agents: may be used for bladder and bowel problems. Non-pharmacologic solutions may be used. |
| UTI | Antibiotic agents (when appropriate) are used for UTI that superimposes over underlying neurologic dysfunction. |
Nursing Process
Assessment
- Address neurologic deficits and the impact of the disease on the patient and family.
- Observe mobility and balance for fall risk assessment. Perform assessments both for when the patient is rested and when the patient is fatigued.
- Assess for weakness, spasticity, visual impairment, incontinence, and disorders of swallowing and speech.
- Additional areas of assessment include quality of life, coping, adherence to medication, and patient goals.
Diagnosis
- Impaired mobility associated with weakness, muscle paresis, spasticity, increased weight
- Risk for fall associated with sensory and visual impairment, lower extremity weakness
- Fatigue associated with insufficient energy
- Difficulty coping associated with uncertainty of course of MS
- (Collaborative) Constipation or fecal incontinence
- (Collaborative) Communication issues and potential for aspiration related to cranial nerve involvement
- (Collaborative) Cognitive changes
Planning, Goals, Intervention
- Promoting Physical Mobility
- Exercise: walking improves gait, especially in relation to loss of proprioception. Assistive devices may also be used. Muscle stretching minimizes contractures; especially for hamstrings, gastrocnemius muscles, hip adductors, biceps, and wrist and finger flexors.
- Minimizing Spasticity and Contractures: warm packs are beneficial, but hot is not advised due to burn risks (especially secondary to sensory loss). Prescribed orthotics help maintain a functional position and reduce contractures. A “stretch-hold-relax” routine is helpful for spasticity. Swimming and stationary bicycling is helpful.
- Activity and Rest: work and exercise just short of fatigue is encouraged. Frequent short rest periods is advised. Exposure to heat increases fatigue and muscle weakness, so air conditioning is recommended.
- Nutrition: many patients with MS are obese or overweight. Corticosteroids used for acute exacerbations contribute to weight gain. Lifestyle changes should be made for weight reduction. Avoidance of alcohol and cigarette smoking are also included.
- Preventing Falls: ataxia produces a risk for falls.
- The patient is instructed to walk with feet apart to widen the base of support and to increase walking stability.
- If proprioception is lost, the patient is instructed to watch their feet while they walk.
- Assistive devices may be required. If gait remains inefficient, the use of a wheelchair or motorized scooter may be the solution. Coordinate with the physical therapist. Wheelchairs can contribute to risk for pressure injuries.
- If intention tremors are noted, wrist weights or neuromodulation devices may be used.
- Managing Fatigue: fatigue is found in 60% to 90% of patients, but etiology remains unclear. It is often the most disabling symptom and the most common reason for cessation of employment. It has been found that decreasing the usage of electronic devices can help sleep quality and reduce fatigue.
- Strengthening Coping Mechanisms: assist patients and families to manage or reduce stress and making appropriate referrals for counseling and support to minimize the adverse effects of dealing with chronic illness.
- Monitor for Complications: 50% of MS cases experience major depression. Suicide risk is twice as high as the general population. Cognitive changes, home functioning, and changes in sexuality can result from progression of MS.
Evaluation
- Improves physical mobility
- Participates in gait training and rehabilitation program
- Establishes a balanced program of rest and exercise
- Uses assistive devices correctly and safely
- Is free of falls
- Monitors self and environment for fall risk factors
- Asks for assistance when necessary
- Reports decreased levels of fatigue
- Identifies strategies to decrease fatigue
- Maintains appropriate sleep hygiene behaviors
- Demonstrates effective coping strategies
- Maintains sense of control
- Modifies lifestyle to fit goals and limitations
- Verbalizes desire to pursue goals and developmental tasks of adulthood
- Demonstrates healthy social interactions
- Participates in meaningful activities
- Understands ways to avoid complications and is free of complications
- Explains reasons for measures to prevent complications